Peripheral gangrene: A rare presentation of systemic lupus erythematosus in a child

Abstract

Patient: Female, 12Final Diagnosis: Antiphospholipid positive SLESymptoms: Gangrene • Raynaud’s phenomenon • autoamputation of the terminal phalanx of the second left hand fingerMedication: Prednisolone • mycophenolate mofetil • captoprilClinical Procedure: Renal Biopsy • treatment of lupus nephritis • control of hypertensionSpecialty: Pediatric rheumatologyObjective:Unusual clinical courseBackground:SLE in children has many manifestations. In several studies on SLE in children, gangrene and Raynaud phenomenon have been described as a rare manifestation of SLE during its course in children.Case Report:We present the case of a 12-year-old girl referred to our center, presenting with peripheral gangrene plus Raynaud’s phenomenon, who proved to have SLE. Our patient was treated with steroids and mycophenolate mofetil. She appeared to respond to this combination judging by the disappearance of the digital cyanosis, appearance of extremity pulses, and return of renal function.Conclusions:This case highlights the importance of precise management and awareness of very rare manifestations of a common disease like SLE. Gangrene can be initial symptom of SLE in children. We recommend SLE evolution in all children with gangrene symptom.

A Rare Case of Digital Ulceration and Gangrene as an Initial Presentation of Systemic Lupus Erythematosus in a Child

“…A study involving 50 adult female SLE patients found that the individuals who developed cutaneous vasculitis were younger when compared to patients who did not develop vasculitis [11]. SLE vasculitis and related digital gangrene can be treated with a variety of drugs, and drug combinations include corticosteroids, immunosuppressant drugs such as mycophenolate mofetil [12], and monoclonal antibodies such as rituximab. According to a study done by Liu et al on 2684 patients with SLE showed Raynaud's phenomenon (RP), elevated serum C-reactive protein (CRP), and lengthy disease duration (> or equal to 4 years) all increased the risk of developing digital gangrene and early treatment with steroids (prednisone > or equal to 1mg/kg/d started within 3 weeks) signi cantly reduced the risks of amputation [13].…”

A Rare Case of Digital Ulceration and Gangrene as an Initial Presentation of Systemic Lupus Erythematosus in a Child

“…There are many drugs that can be used to treat SLE vasculitis and related digital gangrene. Some of these are corticosteroids, immunosuppressant drugs like mycophenolate mofetil [ 14 ], and monoclonal antibodies like rituximab. A study by Liu et al looked at 2,684 SLE patients and found that Raynaud's phenomenon (RP), high serum C-reactive protein (CRP), and having the disease for more than four years all made it more likely that the patients would get digital gangrene.…”

Unveiling Uncommon Manifestations in a Pediatric Patient With Systemic Lupus Erythematosus: A Case Report

“…Cardiac vasculitis in JSLE typically comprises pericarditis, cardiomegaly, valvulitis, and conduction abnormalities. The most common causes of extremity gangrene in SLE are APS and endarteritis [ 11 ]. Differentiating vasculopathy/thrombosis and vasculitis in SLE-associated APS can be difficult and requires histological assessment of cutaneous lesions.…”